Splenomegaly

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: ; Gichoya Judy Wawira [2], Moi University School of Medicine

Synonyms and keywords: Spleen enlargement; enlarged spleen; spleen swelling

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Splenomegaly from Other Diseases

Category Disease Etiology Clinical manifestations Para−clinical findings Gold standard Associated findings
Demography History Symptoms Signs Lab Findings Imaging
Fever Fatigue Weight Abdominal pain Bleeding BP JVP Jaundice Ascites Other CBC Iron LFT PT/PTT BUN/Cr UA Histopathology
WBC Hb Plt
Congestive Cirrhosis[1]
  • Infection
  • Alcohol
  • Autoimmune
Any, more in elderly + ↑↓ + + Nl + +
  • Spider angiomata
  • Palmar erythema
  • Weakness
Nl
  • Dark urine
  • Pancytopenia
  • Regenerating nodules of hepatocytes
  • Fibrosis
  • Deposition of connective tissue between these nodules
  • Small and nodular liver on ultrasound
  • Fibrosis on liver scan
Liver biopsy
  • Hepatic encephalopathy
  • Gynecomastia
  • Hypogonadism
  • Esophageal varices
  • Caput medusa
  • Dupuytren's contracture
Heart failure[2]
  • Coronary artery disease
  • HTN
Elderly
  • Smoking
  • Sedentary lifestyle
  • Obstructive sleep apnea
+ ↑↓ ± +
  • Dyspnea
  • Weakness
  • Chest pain
  • Heart murmur
Nl Nl NA
  • Normal or low ejection fraction on echocardiography
  • Cardiomegaly on chest xray
Echocardiography
  • Pulmonary edema
  • Hyponatremia
  • Elevated BNP
Thrombosis of portal, hepatic, or splenic veins[3]
  • Thrombosis
Any
  • Inflammation
  • Infection
  • Cirrhosis
  • Cancer
+ + + + Nl ± +
  • Weakness
  • Hematemesis
  • Melena
Nl
  • Dark urine
NA
  • Reduced or absent blood flow on doppler ultrasonography
Doppler ultrasonography
  • Portal hypertension
  • Esophageal varices
Malignancy Lymphoma[4]
  • Unknown
  • Mutation
  • Infection
Any
  • Epstein–Barr virus
  • Positive family history
  • Smoking
  • HIV
  • Autoimmune diseases
+ + + + Nl Nl +
  • Lymphadenopathy
  • Itching
  • Night sweats
  • Anorexia
  • Respiratory distress or dyspnea
High ferritin Nl
  • Different histopathological features based on types and stages
  • Reed–Sternberg cell
  • Malignant cells
  • Peritoneal lymphomatosis on CT scan
Lymph node biopsy
  • Primary immune disorder
  • Post-transplant
Leukemia[5]
  • Unknown
  • Mutation
Elderly, male>female + + + + Nl Nl ± + High ferritin Nl
  • Leukemic blasts
  • Leukoerythroblastic features
  • Giant platelets
NA Bone marrow examination + clinical manifestation
Polycythemia Vera[6] Mean age >60 years old
  • Positive family history
+ Nl + Nl Nl
  • Erythromelagia
  • Chest pain
  • Stroke-like symptoms
  • Pruritis
  • Dizziness
  • Visual disturbance
  • Facial plethora
  • Painful erythema
  • Lymphadenopathy
Nl to ↑ Nl to ↑ High ferritin Nl Nl Nl Nl
  • Elevated normochromic, normocytic RBCs
  • Thrombocytosis
  • Rarely immature cells
  • Leukoerythroblastic picture
NA WHO criteria for PV
Multiple myeloma[7]
  • Unknown
  • Mutation
  • Infection
Any, more in adults
  • Exposure to chemicals
  • Radiation
  • Pre-existent blood disorders
+ + + + Nl Nl +
  • Lymphadenopathy
  • Itching
  • Night sweats
  • Anorexia
  • Dyspnea
High ferritin Nl
  • Hemoglobinuria
  • Blast cells
Bone marrow examination
Essential thrombocythemia[8]
  • Acquired mutation of JAK2, CALR, or MPL
Mean age >60 years old, female > male ± + Nl + + Nl Nl
  • Flushing
  • Erythromelalgia
  • Transient ischemic attacks
  • Priapism
  • Decreased visual acuity
↑↑ Nl Nl Nl Nl Nl
  • Increased cellularity
  • Hyperplasia and clustering of megakaryocytes
  • Bone marrow reticulin may be increased with no collagen fibrosis
  • Splenomegaly on abdominal CT
  • VTE on spiral chest CT
  • DVT on ultrasound
Bone marrow biopsy
  • Thrombo-hemorrhagic complications
Primary myelofibrosis[9] Mean age >60 years old, male> female + Nl + + Nl Nl + ↑/↓ Low iron Nl Nl
  • Dark urine
Bone marrow biopsy
  • Thrombo-hemorrhagic complications
  • Variable risk for development of acute leukemia
Primary splenic tumors[10]
Metastatic solid tumors[11] Any, more in adults
  • Exposure to chemicals
  • Radiation
  • Pre-existent blood disorders
+ + + + Nl ± ± Low iron, high ferritin
  • Dark urine
  • Blast cells
Biopsy
Category Disease Etiology Demography History Fever Fatigue Weight Abdominal pain Bleeding BP JVP Jaundice Ascites Other WBC Hb Plt Iron LFT PT/PTT BUN/Cr UA Histopathology Imaging Gold standard Associated findings
Infection Viral Hepatitis
Infectious mononucleosis
Cytomegalovirus
Bacterial Salmonella
Brucella
Tuberculosis
Parasitic Malaria
Schistosomiasis
Toxoplasmosis
Kala-azar Visceral leishmaniasis
Infective endocarditis
Fungal
Inflammation Sarcoidosis
Serum sickness
Systemic lupus erythematosus
Felty syndrome
Category Disease Etiology Demography History Fever Fatigue Weight loss Abdominal pain Bleeding BP JVP Jaundice Ascites Other WBC Hb Plt Iron LFT PT/PTT BUN/Cr UA Histopathology Imaging Gold standard Associated findings
Infiltrative Gaucher disease[12]
Niemann-Pick disease
Amyloidosis
Langerhans cell histiocytosis
Hemophagocytic lymphohistiocytosis
Rosai-Dorfman disease
Hematologic (hypersplenic) states Hemolytic anemia
Sickle cell disease
Granulocyte colony-stimulating factor

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications, and Prognosis

Diagnosis

Treatment

Case Studies

Case #1

Related Chapters

References

  1. Wu Z, Zhou J, Pankaj P, Peng B (December 2012). "Laparoscopic and open splenectomy for splenomegaly secondary to liver cirrhosis: an evaluation of immunity". Surg Endosc. 26 (12): 3557–64. doi:10.1007/s00464-012-2366-5. PMID 22710653.
  2. Gedela M, Khan M, Jonsson O (September 2015). "Heart Failure". S D Med. 68 (9): 403–5, 407–9. PMID 26489162.
  3. Ferri PM, Ferreira AR, Fagundes ED, Liu SM, Roquete ML, Penna FJ (2012). "Portal vein thrombosis in children and adolescents: 20 years experience of a pediatric hepatology reference center". Arq Gastroenterol. 49 (1): 69–76. PMID 22481689.
  4. Ishibashi N, Maebayashi T, Aizawa T, Sakaguchi M, Abe O, Saito T, Tanaka Y (May 2015). "Myelosuppression toxicity of palliative splenic irradiation in myelofibrosis and malignant lymphoma". Hematology. 20 (4): 203–7. doi:10.1179/1607845414Y.0000000192. PMID 25131182.
  5. Quest GR, Johnston JB (December 2015). "Clinical features and diagnosis of hairy cell leukemia". Best Pract Res Clin Haematol. 28 (4): 180–92. doi:10.1016/j.beha.2015.10.017. PMID 26614896.
  6. Chou YS, Gau JP, Yu YB, Pai JT, Hsiao LT, Liu JH, Hong YC, Liu CY, Yang CF, Chen PM, Chiou TJ, Tzeng CH (March 2013). "Leukocytosis in polycythemia vera and splenomegaly in essential thrombocythemia are independent risk factors for hemorrhage". Eur. J. Haematol. 90 (3): 228–36. doi:10.1111/ejh.12064. PMID 23281576.
  7. Grieco A, Manna R, Mancini R, Gambassi G (April 1988). "Massive hepatomegaly following splenectomy for myeloid metaplasia". Am. J. Med. 84 (4): 797. PMID 3400674.
  8. Tefferi A, Barbui T (February 2015). "Polycythemia vera and essential thrombocythemia: 2015 update on diagnosis, risk-stratification and management". Am. J. Hematol. 90 (2): 162–73. doi:10.1002/ajh.23895. PMID 25611051.
  9. Cervantes F, Martinez-Trillos A (May 2013). "Myelofibrosis: an update on current pharmacotherapy and future directions". Expert Opin Pharmacother. 14 (7): 873–84. doi:10.1517/14656566.2013.783019. PMID 23514013.
  10. Adachi K, Ui M, Nojima H, Takada Y, Enatsu K (August 2011). "Isolated splenic peliosis presenting with giant splenomegaly and severe coagulopathy". Am. J. Surg. 202 (2): e17–9. doi:10.1016/j.amjsurg.2010.10.002. PMID 21810495.
  11. Huprikar NA, Kurtz MT, Mount CA (October 2013). "Massive splenomegaly and lymphopenia: a unique case of obstructive shock". BMJ Case Rep. 2013. doi:10.1136/bcr-2013-201643. PMC 3822246. PMID 24172780.
  12. Ayto R, Hughes DA (2013). "Gaucher disease and myeloma". Crit Rev Oncog. 18 (3): 247–68. PMID 23510067.